Anti-D-3-phosphoglycerate dehydrogenase (PGDH) antibodies

Autoimmune liver diseases are a group of rare chronic liver disorders caused by immune dysregulation that leads the immune system to attack hepatic structures. Autoimmune hepatitis (AIH) is a chronic inflammatory liver condition characterised by persistent hypertransaminasemia, hypergammaglobulinemia (high levels of IgG) and circulating autoantibodies.

AIH may occur in association with or overlap with other liver diseases, including primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), drug-induced liver injury (DILI) or viral hepatitis, complicating the clinical and diagnostic picture.

Autoimmune hepatitis is classified into three subtypes based on the autoantibody profile:
A. Type 1 AIH: the most common form, can affect both children and adults; defined by the presence of antinuclear antibodies (ANA) and/or anti-smooth muscle antibodies (ASMA);
B. Type 2 AIH: more frequent in paediatric/adolescent patients; characterized by antibodies to LKM1 (liver–kidney microsomal type 1) and/or LC1 (liver cytosol type 1);
C. Type 3 AIH: more recently proposed; defined by anti-SLA/LP (soluble liver antigen/liver–pancreas antigen) antibodies. There is ongoing debate as to whether this should be considered as a separate entity or a variant of type 1 AIH.

Anti-PGDH antibodies are specific for AIH; their clinical relevance is due to the fact that they are detectable in up to 80% of untreated AIH patients and 61% of corticosteroid-treated AIH patients.